The Collis-Nissen Procedure for Long-Gap Esophageal Atresia
Introduction
Esophageal atresia (EA) is a congenital obstruction of the esophagus that is diagnosed either prenatally or within the first hours of life. EA is most commonly diagnosed together with a tracheoesophageal fistula, although these two conditions can also occur as separate anomalies [1]. Several anatomical classification systems exist, but this article uses the classification proposed by the American physician R. E. Gross in 1953 (Figure 1).
EA may be suspected prenatally when ultrasonography reveals polyhydramnios and absence of the gastric bubble. The absence of the gastric bubble can also be observed on X-ray after birth. In the presence of esophageal obstruction, the first sign after birth is frothy saliva from the mouth [2]. In Lithuania, nasogastric tube insertion is still commonly performed in newborns, and the anomaly is therefore often suspected when the tube encounters the blind-ending upper esophageal pouch. To confirm the diagnosis, an X-ray examination with a contrast-filled tube is performed. Unfortunately, the exact causes of this congenital defect remain unknown, and therefore no specific preventive measures are available [3].
The surgical method and treatment outcomes largely depend on the type of EA and associated anomalies. The greatest challenges arise when there is a long (more than 2.5 cm) or very long (more than 3.5 cm) gap between the ends of the esophagus. The treatment algorithm for such newborns, when early primary esophageal reconstruction is not possible, remains a matter of debate [4, 6, 10].
Numerous methods of esophageal elongation before delayed primary esophageal reconstruction have been described, as have esophageal replacement procedures when elongation is unsuccessful. It is generally agreed that the patient's own esophagus is preferable to even the best artificial substitute [12–15]. Therefore, we present a successful modified Collis-Nissen esophageal reconstruction technique for cases involving a very long gap between the esophageal ends.
Clinical Cases
First clinical case. A premature male newborn was born at 35 weeks of gestation, weighing 2,628 g. He was diagnosed with type B EA (Figure 1), with a proximal tracheoesophageal fistula (TEF). On contrast X-ray examination, the end of the tube was visible at the level of the third thoracic vertebra (Th). Multiple other congenital anomalies were identified: 13 pairs of ribs, agenesis of the corpus callosum, patent foramen ovale, and patent ductus arteriosus. The patient underwent surgery on the second day of life: the TEF was ligated and a gastrostomy was created. The gap between the esophageal ends was determined to be 7 cm.
On the third day of life, peritonitis caused by gastrostomy dehiscence was diagnosed. The stomach was fixed, and the subsequent postoperative course was uneventful. For further care and development, the patient was transferred to the children's department of the city hospital and was later cared for at home by his mother. The patient was fed through the gastrostomy, while saliva was continuously suctioned from the blind-ending upper esophageal pouch. At 3 months of age, he returned for evaluation. After contrast material was injected into the stomach, the distance between the esophageal ends was assessed as 5 cm.
At 7 months of age, the boy underwent mediastinotomy and laparotomy, Collis-Nissen gastroplasty and fundoplication, and esophageal reconstruction. The lower end of the esophagus was lengthened to 8 cm. On the sixth postoperative day, anastomotic dehiscence (Figure 2), mediastinitis, and sepsis were diagnosed.
Repeat mediastinotomy was performed, and the mediastinum was drained. The esophagus healed, and after some time the boy began to receive feeding through the dilated esophagus; the dilator was subsequently removed. Esophageal dilation was successfully performed.
The family of the boy, who is now 5 years old, later emigrated from Lithuania to Scandinavia. Since then, the patient's condition has been monitored at a hospital in that country.
Second clinical case. A full-term male newborn was born at 37 weeks of gestation, weighing 3,150 g. He was diagnosed with type C EA (Figure 1), with a distal TEF. A 10 cm tube was inserted into the esophagus. As the patient was also diagnosed with multiple other anomalies—rectal and anal atresia, congenital heart defects (atrial septal defect and patent ductus arteriosus), and hydronephrosis—VACTERL syndrome was diagnosed after consultation with geneticists.
The patient underwent surgery on the first day of life: the TEF was closed, and a gastrostomy and sigmoidostomy were created. The gap between the esophageal ends was determined to be 6 cm. The patient remained in the hospital, with active suctioning of saliva from the blind-ending upper esophageal pouch, and was fed through the gastrostomy.
At 2.5 months of age, the distance between the esophageal ends was evaluated, during which the lower end of the esophagus was perforated. Mediastinotomy was performed, the esophagus was sutured, and the mediastinum was drained.
At 4 months of age, the patient underwent mediastinotomy and laparotomy, Collis-Nissen gastroplasty and fundoplication, and esophageal reconstruction. The lower esophageal segment was lengthened by 5 cm. On the fifth postoperative day, anastomotic dehiscence, mediastinitis, sepsis, and developing cicatricial stenosis of the esophagus were diagnosed. Antibiotic therapy was prescribed, and esophageal bougienage was performed.
At 6 months of age, because of recurrent evagination of the distal sigmoid stoma, sigmoidostomy correction was performed; at 10 months, A. Peña's proctoplasty was performed; and at 12 months, the sigmoidostomy was closed.
For esophageal and anal stenoses, bougienage was performed every 2 weeks. Rehabilitation treatment was prescribed. At 3 years of age, the patient was diagnosed with iron-deficiency anemia caused by reduced gastrointestinal absorption. At 4 years of age, nephrolithiasis was diagnosed, which did not require treatment. Spinal X-rays showed no abnormalities. The boy, currently 4 years old, is being cared for in a children's foster home.
Third clinical case. A premature male newborn was born at 35 weeks of gestation, weighing 2,620 g. The patient was diagnosed with EA with a distal TEF (type C, Figure 1) and a bronchopleuroesophagocutaneous fistula. He underwent surgery on the first day of life: the TEF was repaired through a right mediastinotomy, and a gastrostomy was created. The aortic arch was located on the right side. The gap between the esophageal ends was determined to be 4 cm (Figure 3).
In the third month of life, the patient underwent another operation. A persistent 3 cm gap was confirmed, and the Foker technique was applied. One month later, the lower esophageal segment recanalized and a mediastinal abscess developed, requiring mediastinotomy and drainage of the abscess. Eleven days later, the patient began regurgitating saliva mixed with milk through the mouth. Recanalization of the lower esophagus and TEF was suspected, and another mediastinotomy and drainage procedure was performed. Air leakage into the chest was identified at several sites.
Because the bronchopleuroesophagocutaneous fistula failed to close spontaneously for a prolonged period, mediastinotomy was performed, the lower esophageal segment was mobilized, and a lower esophagostomy was created posteriorly. The patient was fed through the esophagostomy, and the gastrostomy was therefore closed.
At 7 months of age, following mediastinotomy and laparotomy, Collis-Nissen gastroplasty and fundoplication and esophageal reconstruction were performed. The lower esophageal segment was lengthened by 8 cm. On the second postoperative day, pneumothorax was diagnosed; thoracostomy was performed and a drain was inserted. On the fifth postoperative day, an anastomotic leak, mediastinitis, and hydropneumothorax were detected. The patient was successfully treated conservatively. On the 15th postoperative day, the patient pulled out the tube; laparotomy was performed, and a gastrostomy for feeding was created again.
Periodic esophageal dilations were performed because of cicatricial stenosis in the lower third of the esophagus. At 10 months of age, the gastrostomy was removed and oral feeding was initiated. The patient underwent rehabilitation for hip contracture and developmental delay. At 1.5 years of age, the patient had no complaints, swallowing and appetite were good, and there was no cough. The boy is now almost 3 years old.
Discussion
American surgeon J. E. Foker classified EA into the following groups according to the gap between the esophageal ends: short (up to 1 cm), medium (1–2.5 cm), long (over 2.5 cm), and very long (over 3.5 cm). All three of the patients described in our series had EA with a very long gap. This type of EA is a very rare congenital anomaly, occurring in 1 in 70,000 newborns (for comparison, EA with a shorter gap occurs in 1 in 3,000–4,000 newborns) [1].
In patients with a very long gap between the esophageal ends, i.e., >3.5 cm, early primary esophageal reconstruction cannot be performed [2]. The main treatment for very long-gap EA remains esophageal replacement surgery, including gastric transposition into the chest, creation of an esophagus from the greater curvature of the stomach (gastric tube), and small or large intestinal interposition [3]. Unfortunately, these procedures are highly complex and are associated with frequent early complications, such as graft ischemia, anastomotic dehiscence, and strictures, as well as late complications, including graft twisting, swallowing disorders, gastroesophageal reflux, peptic ulcers, malabsorption [2, 4, 5], and tracheomalacia [6]. Furthermore, aesthetic outcomes remain a challenging issue [2].
It is therefore generally agreed that a child's own esophagus is preferable to even the best artificial substitute [1]. For this reason, increasing attention in recent years has been focused on delayed primary esophageal reconstruction. During the first days of life, only TEF repair, if present, and creation of a gastrostomy for feeding are recommended. It is important to determine the distance between the esophageal ends: the greater the distance, the greater the tension on the anastomotic sutures. The main surgical principle is to avoid this tension, and therefore methods of reducing the distance between the esophageal ends continue to be explored.
Since 1965, various methods of esophageal elongation have been used, including spontaneous esophageal growth (during the first months of life, the gap between the esophageal ends tends to decrease because the newborn's esophagus grows faster than the chest), elongation of the proximal end or both ends (the distal end through the gastrostomy), Rehbein's method using suture bougienage, and approximation of the esophageal ends by introducing electromagnets into them [3, 7]. Esophageal elongation can also be performed surgically, including esophagomyotomy of the upper esophageal segment (A. Livaditis, 1973), an extrathoracic multistage esophageal lengthening technique with esophagostomy (K. Kimura, 1994), creation of a flap from the upper esophageal segment (M. E. Gough, 1980), and elongation using sutures (Foker's technique). After several months, as the esophagus grows and its ends approximate, delayed primary esophageal reconstruction is performed by connecting the esophageal ends end-to-end.
In the patients described here, after very long-gap esophageal atresia was diagnosed, delayed primary esophageal reconstruction was selected as the treatment strategy. A modified Collis-Nissen gastroplasty and fundoplication were used to lengthen the esophagus. Although this method is commonly used to treat gastroesophageal reflux, short esophagus, and congenital esophageal stenosis [8], it can also be applied in the treatment of esophageal atresia with a long gap between the esophageal ends (Figure 4).
When using this gastroplasty and fundoplication technique, it is important to prepare the patient appropriately for surgery and create a gastrostomy for feeding. The method has several disadvantages: during growth, the better blood supply to the distal esophageal segment may result in enlargement of the stomach; the child cannot swallow food after surgery; there is a high risk of aspiration pneumonia; and the newly formed "esophagus" produces gastric juices. However, the main advantage of this method is the ability to select the desired length of the newly formed esophagus. In all three cases, this technique successfully lengthened the lower esophageal segment to 8 cm. This is consistent with data reported in the literature [9]. Furthermore, because of the good blood supply to the stomach, necrosis is rare. In comparison, graft ischemia occurs more frequently with other methods of esophageal replacement [10–12].
Although all three patients developed anastomotic leakage, mediastinitis, and sepsis following delayed primary esophageal reconstruction, similar findings have been reported in the medical literature [1, 2, 13]. One patient developed cicatricial esophageal stenosis; however, according to various authors, this complication occurs in 31.3–100% of patients even after delayed primary esophageal reconstruction. The literature also reports that this treatment strategy is more frequently associated with gastroesophageal reflux (40–75%) [2, 7, 14]. However, by performing Nissen fundoplication in our patients, these complications were successfully prevented. All three patients underwent successful periodic esophageal dilations. The need for such procedures has also been described in the literature [5, 15].
Compared with other methods of esophageal replacement, Collis-Nissen gastroplasty and fundoplication proved to be a better method in these cases because it avoided graft twisting, peptic ulcers, and swallowing disorders [1, 2, 11, 16]. Two of our patients had no such complaints, while the third could not be interviewed. One of the boys was found to have reduced gastrointestinal absorption and iron-deficiency anemia. Malabsorption syndrome is frequently described in the literature following other reconstructive esophageal procedures [1].
Summary
By choosing Collis-Nissen gastroplasty and fundoplication as a method of delayed primary esophageal reconstruction, it is possible to preserve the native esophagus even when there is a very long gap between the esophageal ends. This technique represents a good alternative to other reconstructive esophageal procedures: the desired length can be selected, the risk of necrosis is low, and gastroesophageal reflux can be prevented in advance. Long-term treatment outcomes in these patients are good.
Source: Pediatrics
